Purpose: To report the management of complicated advanced familial exudative vitreoretinopathy in a predominantly young population.
Methods: This retrospective study was performed on 34 eyes of 25 patients with severe complications of advanced familial exudative vitreoretinopathy, including retinal detachment, corneal opacity, shallow or flat anterior chamber, cataract, posterior pupillary adhesion, secondary glaucoma, vitreous hemorrhage, and preretinal hemorrhage. Preoperative and postoperative clinical information was reviewed.
Results: The average age of the patients was 3.52 ± 5.94 years. Of the 34 eyes, 22 underwent lensectomy, 9 underwent lensectomy combined with vitrectomy, 2 underwent staged lensectomy and vitrectomy, and 1 underwent lens-sparing vitrectomy. After surgery, the shallow or flat anterior chamber became normal in 26/28 eyes; corneal opacity disappeared or improved in 10/22 eyes; and secondary glaucoma was controlled in 22/24 eyes. Among the 12 eyes operated by vitrectomy, the retina was attached in 5 eyes and partly attached in 7. Final visual acuity ranged from no light perception to 30/200 (n = 17). All 5 eyes with preoperative and postoperative visual acuity records showed improvement.
Conclusion: Surgical intervention is recommended to resolve complications of advanced familial exudative vitreoretinopathy and to preserve visual function. Staged lensectomy and vitrectomy is an alternative for advanced familial exudative vitreoretinopathy with corneal complications and/or vascularly active fibrovascular proliferation.